Bonten EJ - Search Results

1

Short-term, high dose enzyme replacement therapy in sialidosis mice.

Wang D, Bonten EJ, Yogalingam G, Mann L, d'Azzo A. Wang D, et al. Among authors: bonten ej. Mol Genet Metab. 2005 Jul;85(3):181-9. doi: 10.1016/j.ymgme.2005.03.007. Epub 2005 Apr 25. Mol Genet Metab. 2005. PMID: 15979029

2

Targeting macrophages with baculovirus-produced lysosomal enzymes: implications for enzyme replacement therapy of the glycoprotein storage disorder galactosialidosis.

Bonten EJ, Wang D, Toy JN, Mann L, Mignardot A, Yogalingam G, D'Azzo A. Bonten EJ, et al. FASEB J. 2004 Jun;18(9):971-3. doi: 10.1096/fj.03-0941fje. Epub 2004 Apr 14. FASEB J. 2004. PMID: 15084520

3

Neuraminidase 1 is a negative regulator of lysosomal exocytosis.

Yogalingam G, Bonten EJ, van de Vlekkert D, Hu H, Moshiach S, Connell SA, d'Azzo A. Yogalingam G, et al. Among authors: bonten ej. Dev Cell. 2008 Jul;15(1):74-86. doi: 10.1016/j.devcel.2008.05.005. Dev Cell. 2008. PMID: 18606142 Free PMC article.

4

Chaperone-mediated gene therapy with recombinant AAV-PPCA in a new mouse model of type I sialidosis.

Bonten EJ, Yogalingam G, Hu H, Gomero E, van de Vlekkert D, d'Azzo A. Bonten EJ, et al. Biochim Biophys Acta. 2013 Oct;1832(10):1784-92. doi: 10.1016/j.bbadis.2013.06.002. Epub 2013 Jun 12. Biochim Biophys Acta. 2013. PMID: 23770387 Free PMC article.

5

Lysosomal multienzyme complex: pros and cons of working together.

Bonten EJ, Annunziata I, d'Azzo A. Bonten EJ, et al. Cell Mol Life Sci. 2014 Jun;71(11):2017-32. doi: 10.1007/s00018-013-1538-3. Epub 2013 Dec 15. Cell Mol Life Sci. 2014. PMID: 24337808 Free PMC article. Review.

6

Systemic and neurologic abnormalities distinguish the lysosomal disorders sialidosis and galactosialidosis in mice.

de Geest N, Bonten E, Mann L, de Sousa-Hitzler J, Hahn C, d'Azzo A. de Geest N, et al. Hum Mol Genet. 2002 Jun 1;11(12):1455-64. doi: 10.1093/hmg/11.12.1455. Hum Mol Genet. 2002. PMID: 12023988

7

Protective protein/cathepsin A rescues N-glycosylation defects in neuraminidase-1.

Wang D, Zaitsev S, Taylor G, d'Azzo A, Bonten E. Wang D, et al. Biochim Biophys Acta. 2009 Apr;1790(4):275-82. doi: 10.1016/j.bbagen.2009.01.006. Biochim Biophys Acta. 2009. PMID: 19714866 Free PMC article.

8

A point mutation in the neu-1 locus causes the neuraminidase defect in the SM/J mouse.

Rottier RJ, Bonten E, d'Azzo A. Rottier RJ, et al. Hum Mol Genet. 1998 Feb;7(2):313-21. doi: 10.1093/hmg/7.2.313. Hum Mol Genet. 1998. PMID: 9425240

9

Novel mutations in lysosomal neuraminidase identify functional domains and determine clinical severity in sialidosis.

Bonten EJ, Arts WF, Beck M, Covanis A, Donati MA, Parini R, Zammarchi E, d'Azzo A. Bonten EJ, et al. Hum Mol Genet. 2000 Nov 1;9(18):2715-25. doi: 10.1093/hmg/9.18.2715. Hum Mol Genet. 2000. PMID: 11063730

10

Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization.

Bonten EJ, Campos Y, Zaitsev V, Nourse A, Waddell B, Lewis W, Taylor G, d'Azzo A. Bonten EJ, et al. J Biol Chem. 2009 Oct 9;284(41):28430-28441. doi: 10.1074/jbc.M109.031419. Epub 2009 Aug 7. J Biol Chem. 2009. PMID: 19666471 Free PMC article.

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